Pediatric Hirschsprung Disease Treatment & Management

Updated: Aug 09, 2016
  • Author: Holly L Neville, MD; Chief Editor: Carmen Cuffari, MD  more...
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Treatment

Medical Care

See the list below:

  • If a child with Hirschsprung disease has symptoms and signs of a high-grade intestinal obstruction, initial therapy should include intravenous hydration, withholding of enteral intake, and intestinal and gastric decompression.

  • Decompression can be accomplished through placement of a nasogastric tube and either digital rectal examination or normal saline rectal irrigations 3-4 times daily.

  • Administer broad-spectrum antibiotics to patients with enterocolitis.

  • Immediately request surgical consultation for biopsy confirmation and treatment plan.

  • While awaiting surgical intervention in the event of a planned single-stage pull-through procedure, the baby should receive scheduled vaccinations.

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Surgical Care

The surgical options vary according to the patient's age, mental status, ability to perform activities of daily living, length of the aganglionic segment, degree of colonic dilation, and presence of enterocolitis.

Surgical options include leveling colostomy, which is a colostomy at the level of normal bowel; a staged procedure with placement of a leveled colostomy followed by a pull-through procedure; or a single-stage pull-through procedure. The single-stage pull-through procedure may be performed with laparoscopic, open, or transanal techniques. This procedure can be performed at the time of diagnosis or after the newborn has had rectal irrigations at home and has passed the physiologic nadir. Colostomy followed by pull-through procedure is generally reserved for those patients who present with sepsis due to enterocolitis, massive distention of ganglionic bowel prohibiting pull-through procedure, or are otherwise not medically suitable for the pull-through procedure.

The ability to perform a single-stage pull-through procedure largely depends on the availability, experience, and capabilities of the staff pathologist because aganglionic intestine must not be in the pull-through segment.

Recurrent postoperative enterocolitis may require treatment. Current therapeutic options include rectal dilations, application of topical nitric oxide, posterior myotomy/myectomy, [7] , injection of botulinum toxin, or repeat operation in the event of refractory obstructive symptoms or repeated enterocolitis. [8, 9]

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Diet

A special diet is not required. However, preoperatively and in the early postoperative period, infants on a nonconstipated regimen, such as breast milk, are more easily managed.

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Activity

Postoperatively, patients may return to their normal physical activities.

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